多系统萎缩的诊断与治疗

Diagnosis and therapy of multiple system atrophy

多系统萎缩是一种散发性、快速进展的神经系统退行性疾病,临床表现为以自主神经功能障碍、小脑性共济失调、帕金森综合征及锥体束受损为主的组合症状与体征。过去10年的研究进展提示多系统萎缩与其他神经系统退行性疾病均归于α共核蛋白病。尽管对其诊断在很大程度上仍需基于临床经验,但一些研究进展有助于早期诊断,尤以神经影像学进展显著,并据此修订了多系统萎缩的诊断标准。统一多系统萎缩评价量表的可靠性和有效性已经得到验证,可对多系统萎缩患者进行半定量性临床评价。本文针对目前临床治疗常规,以及一些神经保护治疗和中医辨证施治方法进行总结。

Multiple system atrophy （MSA） is a sporadic and rapidly progressive neurodegenerative disorder characterised clinically by any combination of autonomic, cerebellar ataxia, parkinsonian, and pyramidal signs. Over the past 10 years, substantial progress has been achieved to establish MSA as an ct- synucleinopathy along with other neurodegenerative diseases. Although the diagnosis of this disorder is largely based on clinical expertise, some investigations have been proposed to assist in early differential diagnosis, especially neuroimaging examination, which have resulted in revised diagnostic criteria. The UMSARS is a reliable and valid scale for semiquantitative clinical assessments of MSA patients. An outline of the rationale for managing symptomatic deterioration in MSA is provided including novel neuroprotective therapeutic approaches, together with the treatment of traditional Chinese medicine.